bl-4162a and Acute-Disease

The last four years have seen an explosion in our understanding of the myeloproliferative neoplasms. Important and often unexpected insights into the molecular mechanisms responsible for these disorders have been accompanied by the development of new diagnostic tests and by an improved understanding of the relationship between the different disease entities. This review will focus on recent developments in the pathogenesis and management of essential thrombocythemia with a particular emphasis on its phenotypic overlap with polycythemia vera and primary myelofibrosis.

Topics: Acute Disease; Aged; Aspirin; Clone Cells; Disease Management; Disease Progression; Humans; Hydroxyurea; Interferon-alpha; Janus Kinase 2; Leukemia, Myeloid; Middle Aged; Models, Biological; Mutation, Missense; Polycythemia Vera; Primary Myelofibrosis; Prognosis; Protein Kinase Inhibitors; Quinazolines; Thrombocythemia, Essential; Thrombophilia

Topics: Acute Disease; Alkylating Agents; Aspirin; Cardiovascular Diseases; Clone Cells; Gene Expression; Hemorrhage; Humans; Hydroxyurea; Leukemia, Myeloid; Megakaryocytes; Platelet Aggregation Inhibitors; Quinazolines; Risk; Thrombocythemia, Essential; Thrombophilia

Topics: Abdominal Pain; Acute Disease; Aged; Aspirin; Drug Substitution; Female; Humans; Hydroxyurea; Janus Kinase 2; Mutation; Pancreatitis; Phosphodiesterase Inhibitors; Quinazolines; Thrombocythemia, Essential

Acute leukemia is an uncommon complication of patients with essential thrombocythemia (ET). We describe a patient with ET, who transformed to acute biphenotypic leukemia 4 and 1/2 years after initial ET diagnosis. She had received hydroxyurea, anagrelide, and interferon, in different combinations and varying doses, before leukemic transformation. Acute biphenotypic leukemia was confirmed on bone marrow studies and immunophenotyping. Complete remission (CR) was achieved with induction chemotherapy for acute leukemia. This was followed with consolidation chemotherapy and the patient has remained in CR 9 months after initial induction chemotherapy. To our knowledge, this is a rare event of acute biphenotypic leukemic transformation of a patient with ET.

Topics: Acute Disease; Antineoplastic Combined Chemotherapy Protocols; Chromosome Aberrations; Chromosomes, Human, Pair 20; Cytogenetic Analysis; Female; Follow-Up Studies; Humans; Hydroxyurea; Leukemia; Middle Aged; Quinazolines; Remission Induction; Thrombocythemia, Essential; Treatment Outcome